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Gene Review

DSG3  -  desmoglein 3

Homo sapiens

Synonyms: 130 kDa pemphigus vulgaris antigen, CDHF6, Cadherin family member 6, Desmoglein-3, PVA
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Disease relevance of DSG3

  • Phage lambda genomic clones were obtained containing 4.2 kb upstream of the translation start site of DSG1 and 517 bp upstream of the DSG3 start site [1].
  • The human gene (DSG3) coding for the pemphigus vulgaris antigen is, like the genes coding for the other two known desmogleins, assigned to chromosome 18 [2].
  • We suggest the unambiguously expressed DSG3 protein to be used as a marker for keratoconus [3].
  • Two Dsg are targeted by pathogenic autoantibodies produced in the course of autoimmune bullous skin diseases, Dsg1 in pemphigus foliaceus (PF), and Dsg3 and Dsg1 in pemphigus vulgaris [4].
  • Pemphigus vulgaris (PV) is a potentially lethal skin disease in which epidermal blisters occur as the result of the loss of cell-cell adhesion caused by the action of autoantibodies against a keratinocyte cell surface glycoprotein, the PV antigen (PVA) [2].

Psychiatry related information on DSG3

  • At this reaction time, no further coverage was observed beyond a base/PVA ratio twenty times greater than the stoichiometric ratio [5].

High impact information on DSG3

  • Pemphigus vulgaris (PV) is a life-threatening skin disease in which autoantibodies against a keratinocyte cell surface 130 kd glycoprotein, PV antigen (PVA), cause loss of cell-cell adhesion, with resultant epidermal blisters [6].
  • We used affinity-purified PV IgG to isolate cDNA, containing the entire coding sequence for PVA, from human keratinocyte expression libraries [6].
  • Autoantibodies against the epidermal desmosomal cadherins desmoglein 1 (Dsg1) and Dsg3 have been shown to cause severe to lethal skin blistering clinically defined as pemphigus foliaceus (PF) and pemphigus vulgaris (PV) [7].
  • These results indicate that this baculovirus product has the proper conformation of the authentic PVA and that its conformation is important in pathogenicity of pemphigus [8].
  • To confirm the specificity of this binding we showed that antibodies raised in rabbits against the extracellular portions of PVA also bound desmosomes in these cultures [9].

Chemical compound and disease context of DSG3


Biological context of DSG3

  • Transient transfections with a series of deletion clones indicated that the DSG3 promoter demonstrated keratinocyte-specific expression, as compared with dermal fibroblasts examined in parallel, and fine mapping identified a 30-base pair segment at -200 to -170 capable of conferring epidermal specific expression [15].
  • Primer extension analysis and RNase protection experiments revealed four putative transcription initiation sites for DSG1 at positions -163, -151, -148 and -141, and seven closely linked sites for DSG3, the longest being at -140 relative to the translation start site [1].
  • Analysis of the exon/intron organization of the human desmoglein 4 gene (DSG4) demonstrates that it is composed of 16 exons spanning approximately 37 kb of 18q12 and is situated between DSG1 and DSG3 [16].
  • In this study, the genes for two autoantigens (DSG1 for pemphigus foliaceus and DSG3 for pemphigus vulgaris) were mapped on band q12 of human chromosome 18 by fluorescence in situ hybridization [17].
  • Analysis of 1.9 kb upstream of the translation start site revealed consensus binding sites for transcription factors including Ap-2 and Sp-1, and motifs common to the promoters of other epithelially expressed genes such as keratin 14 and the desmoglein genes DSG1 and DSG3 [18].

Anatomical context of DSG3


Associations of DSG3 with chemical compounds

  • These patients were on prednisolone and immunosuppressives at the time the sera were tested, and it is unclear if the transition from PV to PF is a permanent one or whether it is due to preferential suppression of Dsg3 antibodies below a certain threshold [22].
  • Immunoblot analysis, using ethylenediamine tetra-acetic acid-separated human epidermal extracts, revealed that the patient's serum recognized only a 130-kDa polypeptide which co-migrated with Dsg3 [23].
  • Interestingly, only autoantibodies against the Dsg3 NH2-terminal epitope(s) are able to bind human skin [24].
  • AK23 mAb increased serine phosphorylation of Dsg3 and augmented activation levels of p38 MAPK [25].
  • Of 42 serum samples (25 patients administered carbamazepine, eight patients administered valproic acid and nine healthy volunteers) tested by ELISA, three patients administered carbamazepine showed positive reactivity against both Dsg1 and Dsg3 [26].
  • Cholesterol binding agents such as filipin and nystatin and the tyrosine kinase inhibitor genistein dramatically inhibited Dsg3 internalization [27].

Physical interactions of DSG3


Regulatory relationships of DSG3


Other interactions of DSG3


Analytical, diagnostic and therapeutic context of DSG3


  1. Characterization of the regulatory regions in the human desmoglein genes encoding the pemphigus foliaceous and pemphigus vulgaris antigens. Adams, M.J., Reichel, M.B., King, I.A., Marsden, M.D., Greenwood, M.D., Thirlwell, H., Arnemann, J., Buxton, R.S., Ali, R.R. Biochem. J. (1998) [Pubmed]
  2. The human gene (DSG3) coding for the pemphigus vulgaris antigen is, like the genes coding for the other two known desmogleins, assigned to chromosome 18. Arnemann, J., Spurr, N.K., Buxton, R.S. Hum. Genet. (1992) [Pubmed]
  3. Altered expression of CLC, DSG3, EMP3, S100A2, and SLPI in corneal epithelium from keratoconus patients. Nielsen, K., Heegaard, S., Vorum, H., Birkenkamp-Demtröder, K., Ehlers, N., Orntoft, T.F. Cornea (2005) [Pubmed]
  4. A Truncated Alternative Spliced Isoform of Human Desmoglein 1 Contains a Specific T Cell Epitope Binding to the Pemphigus Foliaceus-Associated HLA Class II DRbeta1*0102 Molecule. Mouquet, H., Farci, S., Joly, P., Maill??re, B., Leblond, J., Drouot, L., Leprince, J., Tonon, M.C., Loiseau, P., Charron, D., Tron, F., Gilbert, D. J. Immunol. (2006) [Pubmed]
  5. Preparation and characterization of alkylated poly(vinyl alcohol) hydrogels using alkyl halides. Duncan, A.C., Sefton, M.V., Brash, J.L. Journal of biomaterials science. Polymer edition. (1996) [Pubmed]
  6. Autoantibodies against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion. Amagai, M., Klaus-Kovtun, V., Stanley, J.R. Cell (1991) [Pubmed]
  7. Pemphigus foliaceus IgG causes dissociation of desmoglein 1-containing junctions without blocking desmoglein 1 transinteraction. Waschke, J., Bruggeman, P., Baumgartner, W., Zillikens, D., Drenckhahn, D. J. Clin. Invest. (2005) [Pubmed]
  8. Absorption of pathogenic autoantibodies by the extracellular domain of pemphigus vulgaris antigen (Dsg3) produced by baculovirus. Amagai, M., Hashimoto, T., Shimizu, N., Nishikawa, T. J. Clin. Invest. (1994) [Pubmed]
  9. Pemphigus vulgaris antigen, a desmoglein type of cadherin, is localized within keratinocyte desmosomes. Kárpáti, S., Amagai, M., Prussick, R., Cehrs, K., Stanley, J.R. J. Cell Biol. (1993) [Pubmed]
  10. Pemphigus vulgaris-IgG causes a rapid depletion of desmoglein 3 (Dsg3) from the Triton X-100 soluble pools, leading to the formation of Dsg3-depleted desmosomes in a human squamous carcinoma cell line, DJM-1 cells. Aoyama, Y., Kitajima, Y. J. Invest. Dermatol. (1999) [Pubmed]
  11. Conformational epitope mapping and IgG subclass distribution of desmoglein 3 in paraneoplastic pemphigus. Futei, Y., Amagai, M., Hashimoto, T., Nishikawa, T. J. Am. Acad. Dermatol. (2003) [Pubmed]
  12. Steady flow and wall compression in stenotic arteries: a three-dimensional thick-wall model with fluid-wall interactions. Tang, D., Yang, C., Kobayashi, S., Ku, D.N. Journal of biomechanical engineering. (2001) [Pubmed]
  13. The potential transmission of infectious agents by semen packaging during storage for artificial insemination. Russell, P.H., Lyaruu, V.H., Millar, J.D., Curry, M.R., Watson, P.F. Anim. Reprod. Sci. (1997) [Pubmed]
  14. Remediation of coal mining wastewaters using chitosan microspheres. Geremias, R., Pedrosa, R.C., Benassi, J.C., Fávere, V.T., Stolberg, J., Menezes, C.T., Laranjeira, M.C. Environmental technology. (2003) [Pubmed]
  15. Cloning of the gene for human pemphigus vulgaris antigen (desmoglein 3), a desmosomal cadherin. Characterization of the promoter region and identification of a keratinocyte-specific cis-element. Silos, S.A., Tamai, K., Li, K., Kivirikko, S., Kouba, D., Christiano, A.M., Uitto, J. J. Biol. Chem. (1996) [Pubmed]
  16. Genetic evidence for a novel human desmosomal cadherin, desmoglein 4. Whittock, N.V., Bower, C. J. Invest. Dermatol. (2003) [Pubmed]
  17. The human genes for desmogleins (DSG1 and DSG3) are located in a small region on chromosome 18q12. Wang, Y., Amagai, M., Minoshima, S., Sakai, K., Green, K.J., Nishikawa, T., Shimizu, N. Genomics (1994) [Pubmed]
  18. Cloning and transcriptional analysis of the promoter of the human type 2 desmocollin gene (DSC2). Marsden, M.D., Collins, J.E., Greenwood, M.D., Adams, M.J., Fleming, T.P., Magee, A.I., Buxton, R.S. Gene (1997) [Pubmed]
  19. Classification, clinical manifestations, and immunopathological mechanisms of the epithelial variant of paraneoplastic autoimmune multiorgan syndrome: a reappraisal of paraneoplastic pemphigus. Nguyen, V.T., Ndoye, A., Bassler, K.D., Shultz, L.D., Shields, M.C., Ruben, B.S., Webber, R.J., Pittelkow, M.R., Lynch, P.J., Grando, S.A. Archives of dermatology. (2001) [Pubmed]
  20. Pemphigus vulgaris antigen (desmoglein 3) is localized in the lower epidermis, the site of blister formation in patients. Amagai, M., Koch, P.J., Nishikawa, T., Stanley, J.R. J. Invest. Dermatol. (1996) [Pubmed]
  21. Lack of mucosal involvement in pemphigus foliaceus may be due to low expression of desmoglein 1. Shirakata, Y., Amagai, M., Hanakawa, Y., Nishikawa, T., Hashimoto, K. J. Invest. Dermatol. (1998) [Pubmed]
  22. Three cases of transition from pemphigus vulgaris to pemphigus foliaceus confirmed by desmoglein ELISA. Ng, P.P., Thng, S.T. Dermatology (Basel) (2005) [Pubmed]
  23. Herpetiform pemphigus showing reactivity with pemphigus vulgaris antigen (desmoglein 3). Kubo, A., Amagai, M., Hashimoto, T., Doi, T., Higashiyama, M., Hashimoto, K., Yoshikawa, K. Br. J. Dermatol. (1997) [Pubmed]
  24. Role of intramolecular epitope spreading in pemphigus vulgaris. Salato, V.K., Hacker-Foegen, M.K., Lazarova, Z., Fairley, J.A., Lin, M.S. Clin. Immunol. (2005) [Pubmed]
  25. Pathogenic monoclonal antibody against desmoglein 3 augments desmoglein 3 and p38 MAPK phosphorylation in human squamous carcinoma cell line. Kawasaki, Y., Aoyama, Y., Tsunoda, K., Amagai, M., Kitajima, Y. Autoimmunity (2006) [Pubmed]
  26. Increased antibody levels to desmogleins 1 and 3 after administration of carbamazepine. Yoshimura, T., Seishima, M., Nakashima, K., Yasuhara, Y., Adachi, S., Kawaguchi, M., Minoura, N., Nakao, T., Kobayashi, J., Yamazaki, F. Clin. Exp. Dermatol. (2001) [Pubmed]
  27. Pemphigus vulgaris IgG-induced desmoglein-3 endocytosis and desmosomal disassembly are mediated by a clathrin- and dynamin-independent mechanism. Delva, E., Jennings, J.M., Calkins, C.C., Kottke, M.D., Faundez, V., Kowalczyk, A.P. J. Biol. Chem. (2008) [Pubmed]
  28. Desmoglein endocytosis and desmosome disassembly are coordinated responses to pemphigus autoantibodies. Calkins, C.C., Setzer, S.V., Jennings, J.M., Summers, S., Tsunoda, K., Amagai, M., Kowalczyk, A.P. J. Biol. Chem. (2006) [Pubmed]
  29. Peptides Targeting the Desmoglein 3 Adhesive Interface Prevent Autoantibody-induced Acantholysis in Pemphigus. Heupel, W.M., Müller, T., Efthymiadis, A., Schmidt, E., Drenckhahn, D., Waschke, J. J. Biol. Chem. (2009) [Pubmed]
  30. Plakoglobin binding by human Dsg3 (pemphigus vulgaris antigen) in keratinocytes requires the cadherin-like intracytoplasmic segment. Roh, J.Y., Stanley, J.R. J. Invest. Dermatol. (1995) [Pubmed]
  31. Type I regulatory T cells specific for desmoglein 3 are more frequently detected in healthy individuals than in patients with pemphigus vulgaris. Veldman, C., Höhne, A., Dieckmann, D., Schuler, G., Hertl, M. J. Immunol. (2004) [Pubmed]
  32. Clustered cadherin genes: a sequence-ready contig for the desmosomal cadherin locus on human chromosome 18. Hunt, D.M., Sahota, V.K., Taylor, K., Simrak, D., Hornigold, N., Arnemann, J., Wolfe, J., Buxton, R.S. Genomics (1999) [Pubmed]
  33. A case of pemphigus vulgaris showing reactivity with pemphigus antigens (Dsg1 and Dsg3) and desmocollins. Hashimoto, T., Amagai, M., Watanabe, K., Dmochowski, M., Chidgey, M.A., Yue, K.K., Garrod, D.R., Nishikawa, T. J. Invest. Dermatol. (1995) [Pubmed]
  34. A study of desmoglein 1 autoantibodies in pemphigus vulgaris: racial differences in frequency and the association with a more severe phenotype. Harman, K.E., Gratian, M.J., Bhogal, B.S., Challacombe, S.J., Black, M.M. Br. J. Dermatol. (2000) [Pubmed]
  35. Characterization of autoantibodies in pemphigus using antigen-specific enzyme-linked immunosorbent assays with baculovirus-expressed recombinant desmogleins. Ishii, K., Amagai, M., Hall, R.P., Hashimoto, T., Takayanagi, A., Gamou, S., Shimizu, N., Nishikawa, T. J. Immunol. (1997) [Pubmed]
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